Sylvian Fissure Meningioma in Childhood: Report of 3 Cases and Review of the Literature.

OBJECTIVE: Sylvian fissure-based meningioma is a rare occurrence during childhood. We report a small series including 3 such cases, all attached to the arachnoid leaflets of the Sylvian fissure. We present a review of the literature and discuss the impact of possibly effective clinical, imaging, and surgical variables on the outcome. METHODS: In a retrospective chart review of children with intracranial meningioma, we encountered 3 cases with Sylvian fissure meningioma operated within the past 32 years. RESULTS: Two girls, 5 and 7 years old, and 1 boy, 7 years of age, presented with epilepsy, headache, diplopia, and hemiparesis. The duration of illness was 3, 4, and 2 months, respectively. All three tumors were attached to the arachnoid layer of the adjacent Sylvian fissure, and they were not dural based. Gross total resection could be achieved, and no recurrences were encountered after 13, 7, and 2 years. CONCLUSION: We report 3 very rare cases of Sylvian arachnoid-based meningiomas. To our knowledge, this is the study with the longest follow-up period (mean 7.3 years, median 7 years). Even though the overall prognosis of pediatric meningiomas may be worse than that of adult illness, we would like to emphasize that the "rare location" of the Sylvian fissure was not an important factor contributing to the outcome in our children.

Primary intraventricular osteosarcoma in a 3-year-old boy: report of a case and review of literature.

INTRODUCTION: Extraskeletal osteosarcoma (ExOS) is a rare and well-known entity. Three to 4% of ExOSs occur in the head and neck region but anecdotally in the central nervous system (CNS). Primary intracranial osteosarcoma (PIOS) can originate from the skull, brain parenchyma, or meninges. CASE PRESENTATION: A 3-year-old boy with history of head trauma 2 weeks before admission is presented harboring an ExOS in the left temporoparietal region. He was operated with the impression of intraventricular meningioma but turned to be a PIOS without any sources in his skeleton. Tumor recurred after 5 months and patient died in 2 weeks with tumor seeding to the brain stem. CONCLUSION: This case is reported to show failure of surgery as the only treatment for these tumors, highlighting the need for more aggressive treatment.

Painful sporadic osteolysis of the parietal bone 'Gorham's disease'.

We report a 62-year old man, with painful osteolysis 'Gorham disease' of skull. To ameliorate pain, large craniotomy and cranioplasty was performed. There was no sign of recurrence after one year. A brief review of the literature is presented.

Predictive value of diffusion-weighted MRI for tumor consistency and resection rate of nonfunctional pituitary macroadenomas.

BACKGROUNDS: Firm tumor consistency is one of the most important factors that impede sufficient removal of pituitary macroademoas via a transsphenoidal approach. The utility of diffusion-weighted (DW) magnetic resonance imaging (MRI) in predicting the tumor consistency and successfulness of transsphenoidal resection was evaluated in this study. METHODS: Thirty consecutive primary cases of nonfunctional pituitary macroadenomas were prospectively enrolled. Conventional and DW MRI were done for all the patients and the apparent diffusion coefficient (ADC) values and the signal intensity of the solid tumor were determined. Intraoperative report of tumor consistency, the degree of fibrosis and percentage of collagen content were documented. The 8 weeks postoperative MRI was used for calculation of the tumor resection rate. RESULTS: The tumor consistency was soft in 10 patients (33.3 %), intermediate in 14 patients (46.7 %) and hard in 6 patients (20 %). The mean collagen content percentage was 10, 23.5 and 66 % (p = 0.009) and the average resection rate was 75, 43 39 % in the three groups respectively (p = 0.001). The mean ADC value was not significantly correlated with the tumor consistency and resection rate. Tumors with isointense to hyperintense signal on DW MRI were more commonly removable by suction and had higher resection rates than those with hypointense signals (p = 0.019). For ADC values within the range of 600-740 × 10(-3) mm(2)/s, a residual volume larger than 20 % of the tumor was more likely. CONCLUSIONS: DW MRI was useful to predict the tumor consistency, collagen content and the chance of removal of pituitary macroadenomas through endoscopic transsphenoidal surgery, and is recommended in the preoperative patient evaluation.

Remarkable improvement of neurological deficits after surgery in patients with Idiopathic spinal cord herniations. The impact of peroperative neuromonitoring. Case reports.

Introduction: Chronic Idiopathic Spinal Cord Herniation (ISCH) is a very rare spinal cord deformation occurring predominantly in thoracic levels. ISCH lead to progressive myelopathy, spastic paraparesis and Brown Séquard syndrome. Research question: We want to hypothesize that a) the herniated segment can regain its function after untethering despite long-term and complete neurologic dysfunction. b) Intraoperative Electrophysiologic Monitoring (IOEPM) may identify intraoperative changes by monitoring specific neural pathways confirming the efficacy of the intervention in the forthcoming cases. Material & method: It is a retrospective review of data of two cases prospectively collected showing improvement of neurological deficit in cases of ISCH in thoracic levels. We describe two patients with progressive neurological deficits due to ISCH who underwent surgery using electrophysiologic monitoring and have been followed to reach remarkable clinical improvement. Results: The spastic paraparesis of the first case improved remarkably after surgery. Complete foot drop of the other case, persistent for 7 months before intervention, improved after the release of the herniated segment of the cord. Peroperative electrophysiological monitoring did not show changes during surgery. Conclusion: We want to hypothesize that the herniated segment can regain its function after untethering despite long-term and complete neurologic dysfunction. Intraoperative Electrophysiologic Monitoring (IOEPM) may confirm the efficacy of the intervention in the forthcoming cases.

Prognostic Factors of the Primary Central Nervous System Lymphoma: Clinical Experience from a Tertiary Care Center in the Middle East.

Aim Primary central nervous system lymphoma (PCNSL) is a rare extra nodal non-Hodgkin's lymphoma. The optimal treatment for PCNSL is still unclear. In this study, we present our experience with management of PCNSL in a tertiary care center in Iran. Methods In this retrospective study, 58 patients with tissue diagnosis of PCNSL were studied. All patients were treated with chemotherapy including intravenous high-dose methotrexate, rituximab and temozolomide and radiotherapy by the same oncologist. Statistical analysis was performed using SPSS. Results The mean overall survival (OS) in this study was 37.4 ± 13.6 months and the mean progression free survival (PFS) was 35.1 ± 9.8 months. The mean time to progression was 15.2 ± 8.79 months among 8 patients who experienced progression in this series. Finding of a positive CSF cytology was not linked with disease progression, while HIV infection and multifocal involvement at initial presentation were strongly linked to a lower PFS. The single most important factor affecting the OS was the histopathologic type of the PCNSL; two of the three patients who died from their disease in this series had non-B cell PCNSL, whereas only one patient with DLBCL died because of brainstem involvement. Conclusion The results of this study show a lower rate of HIV-infection in patients with PCNSL as compared to the series from the western countries. Non-B cell histopathology and HIV-infection were found to be associated with the dismal prognosis.

Development of a comprehensive assessment tool to measure the quality of care for individuals with traumatic spinal cord injuries.

OBJECTIVE: To develop a comprehensive assessment tool to evaluate the Quality of Care (QoC) in managing individuals with traumatic spinal cord injuries (TSCI). METHOD: At first, the concepts of QoC for TSCI were identified by conducting a qualitative interview along with re-evaluation of the results of a published scoping review (conceptualization). After operationalization of indicators, they were valued by using the expert panel method. Afterward, the content validity index (CVI) and content validity ratio (CVR) were calculated and served as cut-offs for indicator selection. Then specific questions were developed for each indicator and classified into three categories: pre-hospital, in-hospital, and post-hospital. Data availability of the National Spinal Cord Injury Registry of Iran (NSCIR-IR) was subsequently used to design questions that represent indicators in an assessment tool format. The comprehensiveness of the tool was evaluated using a 4-item Likert scale by the expert panel. RESULT: Twelve experts participated in conceptualization and 11 experts participated in operationalization phase. Overall, 94 concepts for QoC were identified from published scoping review (87 items) and qualitative interviews (7 items). The process of operationalization and indicator selection led to the development of 27 indicators with acceptable content validity. Finally, the assessment tool contained three pre-hospital, twelve in-hospital, nine post-hospital, and three mixed indicators. Ninety-one percent of experts evaluated the entire tool as comprehensive. CONCLUSION: Our study presents a health-related QoC tool that contains a comprehensive set of indicators to assess the QoC for individuals with TSCI. However, this tool should be used in various situations to establish construct validity further.

Meningiomas in children and adolescents: a meta-analysis of individual patient data.

BACKGROUND: The epidemiological, prognostic, and therapeutic features of child and adolescent meningioma are poorly defined. Clinical knowledge has been drawn from small case series and extrapolation from adult studies. This study was done to pool and analyse the clinical evidence on child and adolescent meningioma. METHODS: Searches of PubMed, Medline, and Embase identified 35 case series of child and adolescent meningioma completed over the past 21 years. Individual patient data were obtained from 30 studies via direct communication with investigators. Primary outcomes were relapse-free survival (RFS) and overall survival. Prognostic variables were extent of initial surgery, use of upfront radiotherapy, age, sex, presence of neurofibromatosis, tumour location, and tumour grade. RFS and overall survival were analysed using Kaplan-Meier survival curves and multivariable Cox regression models. FINDINGS: From a total of 677 children and adolescents with meningioma, 518 were eligible for RFS analysis and 547 for overall survival analysis. Multivariable analysis showed that patients who underwent initial gross-total resection had better RFS (hazard ratio 0·16, 95% CI 0·10-0·25; p<0·0001) and overall survival (0·21, 0·11-0·39; p<0·0001) than those who had subtotal resection. No significant benefit was seen for upfront radiotherapy in terms of RFS (0·59, 0·30-1·16; p=0·128) or overall survival (1·10, 0·53-2·28; p=0·791). Patients with neurofibromatosis type 2 (NF2) had worse RFS than those without neurofibromatosis (2·36, 1·23-4·51; p=0·010). There was a significant change in overall survival with time between patients with NF2 compared with those without neurofibromatosis (1·45, 1·09-1·92; p=0·011); although overall survival was initially better for patients with NF2 than for those without neurofibromatosis, overall survival at 10 years was worse for patients with NF2. Patients with WHO grade III tumours had worse RFS than those with WHO grade I (3·90, 2·10-7·26; p<0·0001) and grade II tumours (2·49, 1·11-5·56; p=0·027). INTERPRETATION: Extent of initial surgical resection is the strongest independent prognostic factor for child and adolescent meningioma. No benefit for upfront radiotherapy was noted. Hence, aggressive surgical management, to achieve gross-total resection, is the initial treatment of choice. In the event of a subtotal resection, repeat resection is recommended to achieve maximum extirpation. Close observation is warranted for patients who have a subtotal resection or who have WHO grade III tumours. Patients without neurofibromatosis should have a minimum 10-year follow-up, whereas patients with NF2 should be considered a special risk category, necessitating life-long follow-up. FUNDING: None.

Primary isolated skull base eosinophilic granuloma confined to the anterior clinoid process: illustrative case.

BACKGROUND: Solitary eosinophilic granuloma (EG) occurs anecdotally in the skull base region, and it has been described in only three previous publications. The authors report the first case of EG of the anterior clinoid process (ACP), which was confined to the ACP and presented with decreased vision. OBSERVATIONS: A 38-year-old woman presented with decreased vision of the left eye of 5 months' duration. Her visual acuity was 3/10, other neurological examinations were intact, and there were no other osseous or soft tissue lesions. The lesion was excised using a left-sided craniotomy and transdural clinoidectomy, decompressing the optic nerve both intra- and extradurally. The lesion was characteristic for EG, and no recurrence was detected after 2 years. LESSONS: EG can be confined to the ACP and impair vision. Imaging studies are sensitive but not specific, and surgical decompression is both diagnostic and treatment oriented. Close observation and even adjuvant therapy may be indicated in similar cases.

Adopting Clinical Practice Guidelines for Pharmacologic Management of Acute Spinal Cord Injury from a Developed World Context to a Developing Global Region.

BACKGROUND: Proper utilization of high-quality clinical practice guidelines (CPGs) eliminates the dependence of patients' outcomes on the ability and knowledge of "individual" health care providers and reduces unwarranted variation in care. The aim of this study was to adapt/adopt two CPGs for pharmacologic management of acute spinal cord injury (SCI) using guideline adaptation methods. METHODS: This study was conducted based on the ADAPTE process. Following establishment of an organizing committee and choosing the health topics, we appraised the quality of the CPGs using the Appraisal of Clinical Guidelines for Research & Evaluation II (AGREE II). Then, the authors extracted and categorized suggestions according to Population, Intervention, Professions, Outcomes and Health care setting (PIPOH). The decision-making process was based on systemic evaluation of each suggestion, utilizing a combination of AGREE II scores, the quality of supporting evidence for or against each suggestion and the triad of feasibility, acceptance and adoptability for the Iranian health-care context. RESULTS: Two guidelines were included in the adaptation process. Based on high-quality of these guidelines and the feasibility and adoptability evaluation of the organizing committee, we decided to adopt the suggestion of both guidelines. Overall, seven suggestions were extracted from the source guidelines. CONCLUSION: This work provides a framework to apply guidelines for acute SCI to the developing regions of the world. Attempts should be made to implement these suggestions in order to improve the health outcomes of Iranian SCI patients.

Prevalence of Abnormal Urodynamic Study Results in Patients with Congenital and Idiopathic Scoliosis and Its Predictive Value for the Diagnosis of Tethered Cord Syndrome: A Single Institution Clinical Study.

STUDY DESIGN: A total of 110 patients with scoliosis were enrolled in this analytical cross-sectional study. PURPOSE: We aimed to compare the urodynamic study (UDS) results of patients with idiopathic scoliosis (ISC) and congenital scoliosis (CSC) and to evaluate the clinical significance of abnormal UDS findings in predicting underlying tethered cord syndrome (TCS). OVERVIEW OF LITERATURE: An abnormal UDS finding is commonly found in patients with CSC. However, there is no consensus regarding its prevalence in patients with ISC. METHODS: Using the STROBE checklist for cross-sectional studies, 110 patients with scoliosis were selected based on our inclusion and exclusion criteria. Among the patients, 76 presented with ISC and 34 with CSC. Demographic data and other details, such as the results of spine radiography, UDS, and magnetic resonance imaging of the spine in both supine and prone positions, were recorded and analyzed. RESULTS: Approximately 50% of patients with CSC had normal UDS findings; 8.8%, mild impairment; and 41.2%, significant abnormalities. Moreover, 67.1% of patients with ISC had normal UDS findings; 9.2%, mild impairment; and 23.7%, significant abnormalities (p =0.166). TCS was identified in 38.2% and 26.3% of patients with CSC and ISC, respectively (p =0.571). In patients with ISC, a significantly abnormal UDS finding indicated that the risk of TCS increased from 26.3% to 50% (odds ratio [OR], 4.2; p =0.009). Meanwhile, in patients with CSC, the risk was almost similar (OR, 0.8; p =0.8). CONCLUSIONS: Even with the absence of subjective urinary symptoms, subclinical urologic impairments can be observed in a significant number of patients with ISC. An abnormal UDS finding can be a sign of underlying spinal cord tethering in a patient with ISC who is a candidate for corrective spine surgery even though it is an independent variant and is not exclusive to candidates for surgery. This finding has high clinical utility for neuro- and ortho-spine surgeons who aim to correct scoliosis (OR, 4.2; p =0.009).

A global, regional, and national survey on burden and Quality of Care Index (QCI) of brain and other central nervous system cancers; global burden of disease systematic analysis 1990-2017.

Primary brain and other central nervous system (CNS) cancers cause major burdens. In this study, we introduced a measure named the Quality of Care Index (QCI), which indirectly evaluates the quality of care given to patients with this group of cancers. Here we aimed to compare different geographic and socioeconomic patterns of CNS cancer care according to the novel measure introduced. In this regard, we acquired age-standardized primary epidemiologic measures were acquired from the Global Burden of Disease (GBD) study 1990-2017. The primary measures were combined to make four secondary indices which all of them indirectly show the quality of care given to patients. Principal Component Analysis (PCA) method was utilized to calculate the essential component named QCI. Further analyses were made based on QCI to assess the quality of care globally, regionally, and nationally (with a scale of 0-100 which higher values represent better quality of care). For 2017, the global calculated QCI was 55.0. QCI showed a desirable condition in higher socio-demographic index (SDI) quintiles. Oppositely, low SDI quintile countries (7.7) had critically worse care quality. Western Pacific Region with the highest (76.9) and African Region with the lowest QCIs (9.9) were the two WHO regions extremes. Singapore was the country with the maximum QCI of 100, followed by Japan (99.9) and South Korea (98.9). In contrast, Swaziland (2.5), Lesotho (3.5), and Vanuatu (3.9) were countries with the worse condition. While the quality of care for most regions was desirable, regions with economic constraints showed to have poor quality of care and require enforcements toward this lethal diagnosis.

The impact of data quality assurance and control solutions on the completeness, accuracy, and consistency of data in a national spinal cord injury registry of Iran (NSCIR-IR).

STUDY DESIGN: Descriptive study. OBJECTIVE: This study aimed to develop and evaluate a systematic arrangement for improvement and monitoring of data quality of the National Spinal Cord (and Column) Injury Registry of Iran (NSCIR-IR)-a multicenter hospital-based registry. SETTING: SCI community in Iran. METHODS: Quality assurance and quality control were the primary objectives in improving overall quality of data that were considered in designing a paper-based and computerized case report. To prevent incorrect data entry, we implemented several validation algorithms, including 70 semantic rules, 18 syntactic rules, seven temporal rules, and 13 rules for acceptable value range. Qualified and trained staff members were also employed to review and identify any defect, inaccuracy, or inconsistency in the data to improve data quality. A set of functions were implemented in the software to cross-validate, and feedback on data was provided by reviewers and registrars. RESULTS: Socio-demographic data items were 100% complete, except for national ID and education level, which were 97% and 92.3% complete, respectively. Completeness of admission data and emergency medical services data were 100% except for arrival and transfer time (99.4%) and oxygen saturation (48.9%). Evaluation of data received from two centers located in Tehran proved to be 100% accurate following validation by quality reviewers. All data was also found to be 100% consistent. CONCLUSIONS: This approach to quality assurance and consistency validation proved to be effective. Our solutions resulted in a significant decrease in the number of missing data.

Neuroscience20 (BRAIN20, SPINE20, and MENTAL20) Health Initiative: A Global Consortium Addressing the Human and Economic Burden of Brain, Spine, and Mental Disorders Through Neurotech Innovations and Policies.

Neurological disorders significantly impact the world's economy due to their often chronic and life-threatening nature afflicting individuals which, in turn, creates a global disease burden. The Group of Twenty (G20) member nations, which represent the largest economies globally, should come together to formulate a plan on how to overcome this burden. The Neuroscience-20 (N20) initiative of the Society for Brain Mapping and Therapeutics (SBMT) is at the vanguard of this global collaboration to comprehensively raise awareness about brain, spine, and mental disorders worldwide. This paper aims to provide a comprehensive review of the various brain initiatives worldwide and highlight the need for cooperation and recommend ways to bring down costs associated with the discovery and treatment of neurological disorders. Our systematic search revealed that the cost of neurological and psychiatric disorders to the world economy by 2030 is roughly $16T. The cost to the economy of the United States is $1.5T annually and growing given the impact of COVID-19. We also discovered there is a shortfall of effective collaboration between nations and a lack of resources in developing countries. Current statistical analyses on the cost of neurological disorders to the world economy strongly suggest that there is a great need for investment in neurotechnology and innovation or fast-tracking therapeutics and diagnostics to curb these costs. During the current COVID-19 pandemic, SBMT, through this paper, intends to showcase the importance of worldwide collaborations to reduce the population's economic and health burden, specifically regarding neurological/brain, spine, and mental disorders.

Sustaining the National Spinal Cord Injury Registry of Iran (NSCIR-IR) in a Regional Center: Challenges and Solutions.

BACKGROUND: The National Traumatic Spinal Cord Injury Registry in Iran (NSCIR-IR), was implemented initially in three hospitals as a pilot phase from 11 Oct 2015 to 19 Jun 2016 and has been active in eight centers from 19 Jun 2016. Poursina Hospital, a trauma care referral center in Rasht, Guilan Province of Iran is one of the registry sites, and has been involved in registering eligible patients since 1 Jan 2016. This study aimed to identify the challenges and solutions for sustaining the NSCIR-IR in a regional center. METHODS: This was a mixed-methods study. For the quantitative analysis, a retrospective observational design was used to measure case capture or case identification rate, mapping cases in the registry against those eligible for registry inclusion amongst the register of hospital admissions. For the qualitative component, data was collected using focus group discussions and semi-structured interviews, followed by thematic analysis. RESULTS: From 19 Jun 2016 to 24 Jan 2018, the proportion of case capture (case identification rate) was 17%. The median time between case identification and data entry to the system was 30.5 d (range: 2 to 193 d). Thematic analysis identified a lack of trained human resources as the most important cause of low case identification rate and delay in data completion. CONCLUSION: Recruitment and education to increase trained human resources are needed to improve case capture, the timeliness of data input and registry sustainability in a regional participating site.

National Guidelines for Cognitive Assessment and Rehabilitation of Iranian Traumatic Brain Injury Patients.

BACKGROUND: Individuals with moderate to severe traumatic brain injury (TBI) often have prolonged cognitive impairments, resulting in long-term problems with their real-life activities. Given the urgent need for evidence-based recommendations for neuropsychological management of Iranian TBI patients, the current work aimed to adapt eligible international guidelines for cognitive assessment and rehabilitation of the TBI patients in Iran. METHODS: The project was led by an executive committee, under the supervision of the Iranian Ministry of Health and Medical Education (MOHME). Following a systematic literature search and selection process, four guidelines were included for adaptation. Clinical recommendations of the source guidelines were tabulated as possible clinical scenarios for 90 PICO clinical questions covering all relevant phases of care. After summing up the scenarios, our initial list of recommendations was drafted according to the Iranian patients' conditions. The final decision-making, with the contribution of a national interdisciplinary panel of 37 experts from across the country, was conducted in two rounds using online and offline survey forms (Round 1), and face-to-face and telephone meetings (Round 2). RESULTS: A total of 63 recommendations in six sections were included in the final list of recommendations, among which 24 were considered as key recommendations. In addition, some of the recommendations were identified as fundamental, meaning that proper implementation of the other recommendations is largely dependent on their implementation. CONCLUSION: Iranian health policy makers and rehabilitation program managers are recommended to address some fundamental issues to provide the necessary infrastructure to set up an efficient cognitive rehabilitation service system.